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视神经脊髓炎谱系障碍中的急性颈段节段性去神经支配

Acute cervical segmental denervation in neuromyelitis optica spectrum disorder.

作者信息

Mingbunjerdsuk Prompan, Katirji Bashar

机构信息

Neurological Institute, University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, OH.

出版信息

J Clin Neuromuscul Dis. 2014 Dec;16(2):90-7. doi: 10.1097/CND.0000000000000060.

Abstract

OBJECTIVES

Neuromyelitis optica (NMO) is an inflammatory demyelinating disease of central nervous system. We report a case of NMO spectrum disorder that developed acute cervical segmental denervation during an episode of transverse myelitis.

METHODS

Case report and a literature review.

RESULTS

A 69-year-old woman presented with right leg weakness, which improved in 2 weeks of intravenous methylprednisolone. Six weeks later, she returned with recurrent transverse myelitis followed by left hand weakness and atrophy. Electromyography showed active denervation on left C8/T1-innervated muscles. Magnetic resonance imaging of thoracic spine showed patchy T2-signal enhancement from T10 to lower cervical spine. NMO antibody was positive. Review of literature revealed 5 cases of cervical transverse myelitis that developed denervation on electromyography.

CONCLUSIONS

Segmental denervation may occur in NMO and variants resulting from severe myelitis extending to involve gray and white matter leads to anterior horn cell loss.

摘要

目的

视神经脊髓炎(NMO)是一种中枢神经系统炎性脱髓鞘疾病。我们报告1例NMO谱系障碍患者,其在横贯性脊髓炎发作期间出现急性颈段失神经支配。

方法

病例报告及文献复习。

结果

一名69岁女性患者出现右腿无力,静脉注射甲泼尼龙2周后症状改善。6周后,她因复发性横贯性脊髓炎再次就诊,随后出现左手无力和萎缩。肌电图显示左侧C8/T1支配的肌肉有失神经活动。胸椎磁共振成像显示从T10至下颈椎有斑片状T2信号增强。NMO抗体呈阳性。文献复习发现5例颈段横贯性脊髓炎患者肌电图显示有失神经支配。

结论

NMO及其变异型可能发生节段性失神经支配,严重脊髓炎累及灰质和白质导致前角细胞丢失。

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